Purpose: To investigate the efficacy of intravitreal bevacizumab injections for treating choroidal neovascularisation (CNV) secondary to pseudoxanthoma elasticum (PXE).
Methods: Patients with active CNV due to PXE received intravitreal bevacizumab (1.5 mg) and were reviewed at monthly intervals. Further treatments were administered depending on disease activity (visual loss of 5 letters or one line, persistent leakage, persistent macular oedema). Baseline and 1-3 monthly follow-up examinations included best corrected visual acuity (BCVA), biomicroscopy, optical coherence tomography (OCT), fluorescein and indocyanine green angiography, fundus autofluorescence and digital fundus photography.
Results: 15 patients (16 eyes) with CNV and PXE were treated. Mean (SD) age was 53 (12.3) years (range 24-72). Mean BCVA at baseline was 20/100 (mean (SD) LogMAR 0.68 (0.51)), improved to 20/63 after the first injection (LogMAR 0.49 (0.45); p = 0.028), and was 20/63 (LogMAR 0.48 (0.48); p = 0.126) at the last follow-up. The mean follow-up time was 8 months. Central retinal thickness decreased significantly from 252 mum at baseline to 214 mum at the last follow-up (p = 0.004) as measured by OCT. Eyes were injected an average of 2.4 times. Categorising patients into two groups (group 1 with only mild changes and group 2 with evident morphological changes in the central macula) revealed that group 1 improved significantly more (LogMAR range 0.41-0.06) than group 2 (LogMAR range 0.80-0.66) (p = 0.014).
Conclusions: The results indicate that intravitreal bevacizumab is effective both functionally and morphologically in treating CNV due to PXE. Best recovery can be achieved in eyes with disease that has not progressed too far and if treatment is initiated at the earliest point possible.